类风湿关节炎与复发性多软骨炎致环杓关节固定的诊断与治疗

目的：探讨类风湿性关节炎（RA）和复发性多软骨炎（RP）的临床特点、诊断与鉴别诊断以及治疗与预后,以提高对RA和RP的认识。方法：回顾性分析11例RA和15例RP患者的临床资料。结果：1991－2000年间收治11例RA和15例RP中,分别有4例（36％）和5例（33％）累及双侧环杓关节。RP中60％有耳软骨炎,33％有鼻软骨炎,47％有呼吸系统受累,部分患者有心血管受累,用皮质激素、免疫抑制和氨苯砜控制症状,4例RA和5例RP患者因呼吸道梗阻行气管切开术,3例RA患者行杓状软骨切除声带外展移位术。15例RP中1例治疗无效,死于呼吸道并发症。结论：RA、RP是自身免疫性疾病,可累及多器官的软骨结构及结缔组织,在耳鼻喉科有表现,易误诊。早期诊断,采用综合治疗是提高疗效改善预后的关键。     

累及气管支气管的复发性多软骨炎患者的护理

复发性多软骨炎(relapsing polychondritis,RP)是一种罕见的以软骨组织炎症为特征的自身免疫性疾病,病变主要累及多器官的软骨,包括耳、鼻、喉、支气管、关节以及肋软骨;亦可累及黏蛋白丰富的组织,如内耳、主动脉、心脏和皮肤[1].据文献[2]报道,就诊时有14%～38%的RP患者有呼吸道受累,而在整个病程中呼吸道受累者占48%～67%.患者常因气管、支气管软骨的缺失,管壁塌陷,导致胸闷、气促、呼吸困难.我科于2000年8月至2008年6月治疗侵犯气管支气管的复发性多软骨炎患者16例,疗效满意,现将护理体会报道如下……     

1例复发性多软骨炎导致困难气道病人的护理

正复发性多软骨炎(relapsing polychondritis,RP)是一种主要以软骨复发性炎症和进行性破坏为特点的自身免疫性疾病,常累及耳、鼻、喉、气管、支气管等富含软骨部位和关节、心脏、内耳、角膜和巩膜等器官。以呼吸系统受累为首发表现的复发性多软骨炎不多,但病程中最终呼吸道受累的可高达50%以上[1]。病人常因气管、支气管软骨的缺失、管壁塌陷导致胸闷、气促、呼吸困难。我科2013年1月成功抢救1例复发性多软骨炎导致困难气道的病人,现将抢救及护理总结如下。     

吸气与呼气胸部CT扫描对儿童复发性多软骨炎的诊断价值

复发性多软骨炎是一种累及全身软骨结构的系统性疾病。临床表现有耳部及鼻腔软骨炎 ,常常合并非侵蚀性关节炎及呼吸道受累。该病在儿童中较为罕见 ,并具有易复发及逐渐加重的特点。由于呼吸道阻塞的并发症 ,其死亡率高达30 %。所以准确评价呼吸道受累情况对该病诊断及监测具有重要意义。　　病例报告 :9岁女孩 ,因反复发作的呼吸困难、喘鸣、多关节疼痛入院。气管切开后抗菌素治疗及平喘治疗两年 ,呼吸道症状仍无好转。在女孩 12岁时 ,出现了双耳软骨炎 ,临床才考虑到复发性多软骨炎的诊断。大剂量免疫抑制剂治疗病人症状无改善。由于呼吸困…     

1例复发性多软骨炎并气管切开患者的居家护理

复发性多软骨炎(Relapsing polychondritis,RP)是一种累及全身多系统的自身免疫性疾病,主要累及耳廓、鼻、喉、气管、支气管及关节等软骨组织,反复炎症可致组织及器官永久性破坏.由于其病因及发病机制尚不明确,几乎半数患者发病初期缺乏典型的临床表现,早期诊断困难,误诊率高.RP一般发病年龄为40～60岁,但也可在儿童期发病.有文献报道:儿童累及呼吸道发生率高,91％的患儿确诊时已有喉部受累,54％的患者已有气管切开[1].由于RP罕见,目前尚无统一的治疗指南,主要根据疾病活动性和器官受累程度进行经验性治疗[2].     

复发性多软骨炎累及气道的护理

复发性多软骨炎(relap sing polychondritis,RP)是一种病因不明、较少见的炎性破坏性病病.其特点是软骨组织复发性、退化性炎症,除典型的耳廓软骨、鼻软骨容易受累外,其他系统或器官如呼吸道、眼部、关节、内耳等也可受累.     

4例儿童复发性多软骨炎的护理

复发性多软骨炎(relapsing polychondritis,RP)是一种较少见的炎症破坏性疾病,其特点是软骨组织复发性退化性炎症,表现为耳、鼻、喉、气管、眼、关节、心脏瓣膜等器官及血管等结缔组织受累。RP为临床少见疾病,1923年首次见于文献报道,称为"多软骨病"。截至2001年,国外报道600多例,截至2003年我国大约报道240例。1976年McAdam提出RP的诊断标准,1979年Damiani等改良了McAdam标准以期能早期发现RP:双耳复发性软骨炎;非侵蚀性关节炎;鼻软骨炎;眼部炎症;喉和     

复发性多软骨炎1例误诊分析

目的提高对复发性多软骨炎的认识,减少误诊。方法报道广西龙潭医院1例诊断复发性多软骨炎患者的临床资料,并进行分析。结果患者曾被误诊为肺部感染及支气管哮喘,经回顾性分析更正诊断为复发性多软骨炎。结论以呼吸道症状为首发表现的复发性多软骨炎需正确诊断,应用糖皮质激素治疗能显著改善症状及预后。     

复发性多软骨炎呼吸道受累特点的临床分析

【目的】总结和评价复发性多软骨炎（RP）呼吸道受累的特点。【方法】从临床表现、胸部影像学、肺功能检查以及支气管镜镜下表现四个方面回顾性分析RP患者呼吸道受累的特点。【结果】RP患者呼吸道受累常见,呼吸道受累的常见症状为咳嗽77．8％（7／9）、气短6627％（6／9）,其他症状包括喘息11．1％（1／9）及声嘶22．2％（2／9）;胸部CT表现气道壁增厚、管腔狭窄达66．7％（6／9）,2例患者出现气道钙化,2例患者伴有阻塞性肺炎;肺功能以阻塞性通气功能障碍为主,且在疾病早期即可出现;纤维支气管镜下所见主要为气道黏膜肿胀、软骨环消失、气道狭窄,病情较重者呼气相气道陷闭。【结论】RP患者呼吸道受累常见,需综合临床表现、胸部影像学、肺功能检查以及支气管镜镜下表现四个方面进行评估,方可确诊。     

一例复发性多软骨炎患者气管切开术后使用人工鼻的护理体会

复发性多软骨炎(Relopsing polychondnits RP)为一种累及全身多处软骨的发作性和进行性炎症,临床可表现为耳、鼻、喉、气管、支气管及关节软骨的软骨炎,并可累及眼、内耳等[1].病因未明,可能是一种在遗传易感性基础上由多种诱发因素刺激导致的自身免疫性疾病[2].其发病率为3.5/100万,男女发病比例无差异,约50％的患者最终可出现呼吸道病变[3],喉和会厌炎症可导致上呼吸道塌陷,形成窒息,需行气管切开术.     

55例Ebstein's畸形的临床分析

目的：探讨Ｅｂｓｔｅｉｎ’ｓ畸形的早期临床诊断。方法：回顾分析５５例确诊为Ｅｂｓｔｅｉｎ‘ｓ畸形患者的临床诊断、治疗效果及随访结果。结果：临床资料表明,临床表现无特异性,超声心动图可准确显示三尖瓣下移的程度及瓣膜发育情况,对明确诊断和手术矫治具有重要价值。３２例经手术矫治者,术后早期死亡５例,获随访的２５例中,仅１例死于右心衰竭。而非手术治疗２３例中,随访了１５例,其中死于右心衰竭８例,猝死１例。     

Anti‐MDA‐5 antibody‐positive clinically amyopathic dermatomyositis with rapidly progressive interstitial lung disease treated with therapeutic plasma exchange: A case series

We present six cases of antimelanoma differentiation‐associated gene 5 antibody (anti‐MDA5‐Ab)‐positive clinically amyopathic dermatomyositis (CADM) with rapidly progressive interstitial lung disease (RP‐ILD), which is known to have a poor prognosis. The outcomes of these cases are described after treatment with therapeutic plasma exchange (TPE). Clinical and therapeutic data for patients with CADM with RP‐ILD were collected retrospectively from medical records. All six patients received early intensive care including high‐dose corticosteroids, intravenous cyclophosphamide, and a calcineurin inhibitor, but lung disease and hypoxia became more severe. TPE was performed over a median of 9.5 sessions (range 3‐14) per patient, and the median duration from admission to TPE was 23 days. Three patients received combined direct hemoperfusion using a polymyxin B‐immobilized fiber column (PMX‐DHP) therapy on successive days to manage acute respiratory failure. Four patients survived and two died due to respiratory failure. In the survival cases, ferritin decreased, and ferritin and KL‐6 were lower at diagnosis. The patients who died had a higher alveolar‐arterial oxygen difference and more severe lung lesions at the time of initiation of TPE. These findings indicate that a combination of conventional therapy and TPE may be useful for improvement of the prognosis of CADM with RP‐ILD at the early stage of onset.     

A Reverse Perfusion Pattern During Technetium-99m Stress Myocardial Perfusion Imaging Does Not Predict Flow Limiting Coronary Artery Disease

BACKGROUND: A reverse redistribution pattern during myocardial perfusion imaging is most widely described using thallium (Tl-201), when stress images exhibit greater perfusion than rest. Technetium (Tc-99 m) radiopharmaceuticals may also yield a reverse perfusion (RP) pattern, but its significance is uncertain. This study tested the hypothesis that RP correlates with the presence and location of flow limiting coronary stenosis(es). METHOD: We reviewed 842 consecutive Tc-99 m tetrofosmin SPECT stress studies performed at a cardiothoracic centre over a 15 month period. 69 (8.2%) demonstrated RP. Thirty-three patients (age 32-79 mean 56, 17 female) had undergone cardiac catheterisation within 12 months of the scan. Correlation was sought between the presence and location of angiographic stenoses and RP pattern. RESULTS: 10/33 (30.3%) had significant (>60%) coronary stenosis(es); 5 single-vessel, 2 two-vessel and 3 three-vessel disease (3VD). Stenosis location correlated poorly with the RP territory (LAD/Anterior 5/17, RCA/Inferior 1/10, Cx/lateral 0/4 (p = 0.57)). Of the 6 patients with a lesion in the RP territory, 3 had 3VD; 2 of these had a simultaneous reversible defect. All 5 patients with previous myocardial infarction had a simultaneous fixed defect. However only 3/12 with co-existent reversible defects had significant disease. CONCLUSION: The reverse perfusion pattern is a poor predictor of flow limiting coronary disease, and does not correlate with stenosis location in those with significant lesions. Such patients should not undergo invasive investigation purely on the basis of this result.     

Systemic manifestations of rheumatoid arthritis, echostructural changes in brachiocephalic arteries

AIM: To define echostructural and hemodynamic features of vascular lesions in rheumatoid arthritis (RA) with systemic manifestations. MATERIAL AND METHODS: Of 125 patients included in the study 70 had RA. They were compared to 40 patients with deforming osteoarthritis (DOA) and 15 patients with atherosclerosis of major cerebral arteries. Extra-articular systemic manifestations were diagnosed in 18 RA (25.7%) patients. Carotid, vertebral arteries and cerebral circulation were studied with ultrasonic duplex scanning on the unit "Vingmed system 5, Norway, 2002". RESULTS: The study group exhibited a significant reduction of linear perfusion velocity (LPV) with increased vascular resistance indexes in the carotids and vertebral arteries on the right and left. It was more evident in RA patients with systemic manifestations. Scanning carotids in RA with SM patients and RA patients without extra-articular manifestations showed differences suggesting that thickness of intima-media complex was larger when systemic manifestations were present. CONCLUSION: The revealed changes in circulation may indicate the systemic process.     

Prognostic value of some serological markers of rheumatoid arthritis in assessment of x-ray outcome of the disease

AIM: To specify significance of some serological markers for diagnosis and x-ray prognosis of rheumatoid arthritis (RA). MATERIAL AND METHODS: A total of 115 RA patients with the disease history not longer than 4 years were examined for serum antibodies to cyclic citrullated peptide (ACCP), rheumatoid factor (RF), antikeratine antibodies (AKA) using enzyme immunoassay and indirect immunofluorescence. RESULTS: ACCP, AKA, IgA-RF, IgM-RF were detected in 79.15%, 47.8%, 64.3%, 89.6% patients, respectively. ACCP was found to be directly correlated with negative x-ray changes in hand and foot joints. ACCP specificity reached 97%, the highest specificity (up to 100%) being in determination of RF and ACCP. CONCLUSION: ACCP is an independent factor increasing significance of x-ray indices and thus allowing prognosis of x-ray outcome of RA.     

Characteristics and outcome of cardiopulmonary resuscitation in hospitalised African children

ObjectiveTo review the characteristics and outcome of cardiopulmonary resuscitation in children at a rural hospital in Kenya.Patients and methodAll children aged 0–14 years who experienced ≥1 episode of respiratory or cardiopulmonary arrest during April 2002–2004 were prospectively identified. Demographic variables, cause of hospitalisation, type and duration of arrest, resuscitation measures taken and outcomes were determined.Results114 children experienced at least one episode of respiratory arrest (RA) or cardiopulmonary arrest (CPA). Cardiopulmonary resuscitation (CPR) was performed on all children. “Do not resuscitate order” (DNR) was given in 15 patients after initial resuscitation. Eighty two patients (72%) had RA and 32 (28%) had CPA. 25/82 (30%) patients with RA survived initial CPR compared to 5/32 (16%) with CPA. Survival at discharge was 22% (18/82) in children who had RA while no one with CPA survived at discharge. The leading underlying diseases were severe malaria, septicaemia and severe malnutrition. Prolonged resuscitation beyond 15 min and receiving adrenaline [epinephrine] (at least one dose of 10 μg/kg IV) were predictive of poor final outcome.ConclusionCardiopulmonary arrest after admission has a very poor prognosis in our hospital. Infectious diseases are the main underlying causes of arrest. If a child fails to respond to the basic tenements of PALS within 15 min then it is unlikely that further efforts to sustain life will be fruitful in hospitals where ventilation facilities are not present.     

An AA-amyloidosis course in patients with rheumatoid arthritis

AIM: To characterize renal amyloidosis in patients with rheumatoid arthritis and stages of amyloid nephropathy. MATERIAL AND METHODS: The trial covered 30 patients (6 males and 24 females) with documented rheumatoid arthritis (RA) complicated with secondary AA-amyloidosis. Amyloidosis diagnosis was confirmed in all the patients morphologically, the samples were studied with the peroxidase immunohistochemical method using specific monoclonal antibodies to SAA. Clinical manifestations of RA were assessed by the disease activity, functional impairment of the joints, x-ray alterations, extraarticular signs of RA, etc. All the patients were examined clinically, total blood count and biochemical tests were made. RESULTS: In 23 (77%) of 30 examinees with RA, proteinuria as the first clinical symptom of AA-amyloidosis emerged with the first 15 years of RA. RA of the second-third degree of activity were diagnosed in 25 (83%) patients, 21 (70%) patients had apparent destructive changes in the joints (x-ray stage III-IV). Severe functional insufficiency of the joints was observed in 25 (83%) patients, deformation of the joints - in 27 (90%) patients. Clinically, renal amyloidosis was characterized by change of stages - from moderate proteinuria to nephrotic syndrome and renal failure. Prognosis of amyloid nephropathy in RA depends on duration of the proteinuric stage: if this stage is short (3 years maximum), the prognosis is worse than in its long duration. CONCLUSION: RA ranks first among causes of secondary AA-amyloidosis. Development of AA-amyloidosis in RA patients is most probable in the first 15 years of the course of the articular process. Amyloidosis is more frequent in patients with severe clinical manifestations of RA.     

Long-term clinical follow-up after rotational atherectomy to coronary arterial stenosis in kawasaki disease

BACKGROUND: Although rotational atherectomy (RA) have been proven to be effective in calcified and non-dilatable lesions in Kawasaki disease, long-term efficacy and safety have not been established. METHOD: To evaluate long-term outcome, follow-up (FU) information was analyzed in 26 patients with Kawasaki disease, or 30 calcified and non-dilatable lesions with adjunctive balloon(BA) dilatation after RA from May 1993 to December 2002. Patient and lesion characteristics were: age 15 +/- 5 (8-28) years(yrs), and male-77%, previous myocardial infarction(MI)-4%, multi-vessel disease-35%, coronary aneurysm-87%. Lesion location was LAD-53%, RCA-40%, LCX-7%. Successful dilatation was achieved in 30 out of 30 lesions (100%) with adjunctive BA after RA. There were no in-hospital major events (Death/MI/coronary artery bypass surgery: CABG) and angiographic complications (perforation, no-reflow, spasm). RESULTS: Six-month FU quantitative coronary angiography(QCA) was performed in 23 patients or 27 lesions (90%). Binary restenosis (defined as > 50% diameter stenosis at FU) was detected in 4 of 27 lesions (15%). Target lesion(TL)-percutaneous coronary intervention(PCI) was performed in 3 of 27 lesions (11%). Neoaneurysm was occurred in 4 of 27 lesions (15%) which was associated with post % diameter stenosis (p = 0.002) and the crack of calcified arch detected by intravascular ultrasoud examination after adjunctive BA dilatation (p = 0.03). Anti-coagulant therapy was performed in all patients with neoaneurysm. Clinical FU data was achieved in all patients. Mean clinical FU interval was 6.1 +/- 3.4 yrs. Event-free survival rates at 5 yrs were as follows: Death 100%, Death/MI/CABG 100%, Death/MI/CABG/TL-PCI 86%, Death/MI/CABG/Any-PCI 86%. There was no any- PCI beyond 6 months. CONCLUSION: RA is an effective treatment in calcified and non-dilatable lesions with Kawasaki disease, associated with high procedural success rates. The safety and efficacy of RA appeared to be sustained at long-term FU.     

Discrepancy in the use of confirmatory tests in patients hospitalized with the diagnosis of chronic obstructive pulmonary disease or congestive heart failure

OBJECTIVE: To determine the prevalence of confirmatory use of spirometry in patients admitted to a tertiary-care facility with the diagnosis of chronic obstructive pulmonary disease (COPD), including those with respiratory failure, and compare that to the use of confirmatory 2-dimensional echocardiography (2-D echo) in patients admitted with the diagnosis of congestive heart failure (CHF), to determine preferential confirmatory testing practices. SETTING: Academic tertiary-care hospital. METHODS: A 6-month retrospective review of charts of patients with a primary or secondary discharge diagnosis of COPD, respiratory failure, and CHF, using the appropriate International Classification of Diseases, Ninth Revision, Clinical Modification codes. Pulmonary function and echocardiography laboratory databases were reviewed to determine if the patients had had spirometry or 2-D echo performed during the 8 years prior to the study period. RESULTS: Five hundred fifty-three patients were discharged with the diagnosis of COPD, and 173 patients (31%) had had spirometry. In contrast, 789 patients had the diagnosis of CHF, and a larger proportion of them (619 patients, 78%) had had 2-D echo (p < 0.001). Only 35% of the patients with respiratory failure and COPD had spirometry performed. There were a total of 219 patients with concomitant diagnoses of COPD and CHF. A majority of them (48%) had a 2-D echo as the only confirmatory test, 74 (34%) had both tests performed, 4 (2%) had spirometry only, and 36 (16%) had neither test performed. Of the patients with a diagnosis of COPD who had spirometry, 30% had spirometry findings consistent with restrictive or normal physiology. CONCLUSIONS: A large proportion of patients hospitalized with the diagnosis of COPD have never had a confirmatory test, including those with presumably advanced disease. Compared to patients with CHF, patients with COPD are less likely to have had the confirmatory test performed, even when both conditions coexist. Many patients with the clinical diagnosis of COPD have an inconsistent physiologic diagnosis. To impact the increasingly important problem of COPD, we must raise awareness of the need to confirm its diagnosis and severity with spirometry.     

类风湿性关节炎患者血清RA-CP，抗CCP 抗体及RF 检测对RA 的实验诊断意义

目的　分析不同血清类型的类风湿性关节炎（RA）患者血清中的类风湿性关节炎特异性抗原瓜氨酸化蛋白（RACP） 的水平，比较RA-CP 与抗环瓜氨酸多肽（CCP）抗体、类风湿因子（RF）在RA 诊断中的价值。方法　用酶联免疫 吸附法（ELISA）检测103 例RA 患者、71 例其他风湿病患者、105 例健康体检者血清中RA-CP，抗CCP 抗体和RF 的 水平。根据抗CCP 抗体和RF 测定结果将RA 患者分为4 种血清类型:抗CCP 抗体阳性/RF 阳性的RA、抗CCP 抗体 阳性/RF 阴性的RA、抗CCP 抗体阴性/RF 阳性的RA、抗CCP 抗体阴性/RF 阴性的RA，分析各种血清类型RA 患者 血清中RA-CP 的水平。结果　RA 组的血清中RA-CP 浓度水平中位数为4.51（1.80~7.49）单位，高于其他疾病组的0.53 （0.40~0.76）单位和健康对照组的0.52（0.35~0.69）单位。RA-CP，抗CCP 抗体和RF 对RA 的敏感度分别为77.7%， 65.0% 和69.9%，RA-CP 对RA 的敏感度高于抗CCP 抗体和RF（P ＜ 0.05）；特异度分别为95.5%，97.2% 和89.2%。在 4 种血清型的RA 中RA-CP 的检出率分别为100.0%（57/57），40.0%（4/10），100.0%（15/15）和19.0%（4/21）。在抗 CCP 抗体阴性的RA 中RA-CP 的检出率为52.8%（19/36）。结论　血清中RA-CP 对RA 的诊断比抗CCP 抗体和RF 具有 更高的敏感度，可提高类风湿性关节炎的检出率。因此RA-CP 可作为RA 临床诊断的新辅助指标。